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Available online 2 September 2021
Aortic events in pregnant patients with Marfan syndrome. Lessons from a multicenter study
Eventos aórticos en el embarazo de pacientes con síndrome de Marfan. Lecciones de un estudio multicéntrico
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Carlos E. Martína,
Corresponding author
carlosestebanmartin@hotmail.com

Corresponding author: Servicio de Cirugía Cardiaca, Hospital Universitario Puerta de Hierro-Majadahonda, Manuel de Falla 1, 28222 Majadahonda, Madrid, Spain.
, Arturo Evangelistab, Gisella Teixidób, Susana Villara, Santiago Serrano-Fiza, Víctor Ospinaa, Susana Mingoc, Vanessa Moñivasc, Daniel Martíneza, Juan Villarreala, Alberto Fortezaa
a Servicio de Cirugía Cardiaca, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, Spain
b Servicio de Cardiología, Hospital Universitario Vall d’Hebron, Barcelona, Spain
c Servicio de Cardiología, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, Spain
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Figures (2)
Tables (4)
Table 1. Prepregnancy clinical characteristics and imaging findings (transthoracic echocardiography, computed tomography, magnetic resonance)
Table 2. Patient characteristics during the peripartum period
Table 3. Characteristics of patients who experienced aortic events during pregnancy
Table 4. Univariate analysis comparing pregnant and nonpregnant women with Marfan syndrome
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Abstract
Introduction and objectives

Pregnancy in women with Marfan syndrome (MS) is associated with an increased risk of aortic events. The clinical evidence on pregnant patients with MS is limited and there is no specific consensus on their optimal management. We report our multicenter experience.

Methods

From January 2004 to January 2020, 632 patients with MS underwent periodic monitoring in Marfan units. During this period, we identified all pregnant women with MS and analyzed the incidence of aortic events during pregnancy and puerperium.

Results

There were 133 pregnancies in 89 women with MS (8 women with prior aortic surgery). There were no maternal deaths, but 5 women had aortic events during the third trimester and puerperium (type A dissections in 2, type B dissection in 1, and significant [≥ 3mm] aortic growth in 2). The aortic event rate was 3.7%. Pregestational aortic diameter≥ 40mm showed a nonsignificant association with aortic events (P=.058). Fetal mortality was 3% and 37.6% of births were cesarean deliveries.

Conclusions

Women with MS have an increased risk of aortic events during pregnancy, especially in the third trimester and postpartum period. Patients with MS and aortic diameters 40mm should be assessed in experienced centers for prophylactic aortic surgery before pregnancy. It is important to provide early diagnosis, prepregnancy study of the aorta, beta-blocker administration, and close monitoring during pregnancy, especially during the last trimester and postpartum.

Keywords:
Marfan syndrome
Pregnancy and postpartum
Aortic dissection
Aortic diameter
Abbreviations:
MS
Resumen
Introducción y objetivos

El embarazo en el síndrome de Marfan (SM) incrementa el riesgo de eventos aórticos. La evidencia clínica actual es escasa y no existe un consenso específico sobre el tratamiento óptimo de estas pacientes. Se presenta nuestra experiencia multicéntrica.

Métodos

Entre enero de 2004 y enero de 2020, 632 pacientes con SM mantuvieron revisiones periódicas en unidades de Marfan. Durante este periodo se identificó a todas las mujeres gestantes y se analizó la incidencia de eventos aórticos durante el embarazo y el puerperio.

Resultados

Se hallaron 133 embarazos de 89 mujeres (8 con cirugía de aorta previa). No hubo mortalidad materna. Cinco mujeres sufrieron eventos aórticos durante el tercer trimestre del embarazo y el puerperio (2 disecciones tipo A, 1 disección tipo B y 2 crecimientos significativos de la aorta (≥ 3mm). La incidencia de eventos aórticos fue del 3,7%. Se evidenció una mayor tendencia a eventos con diámetros aórticos pregestacionales ≥ 40mm (p=0,058). La mortalidad fetal fue del 3%. El 37,6% de los partos se realizaron mediante cesárea.

Conclusiones

Las mujeres con SM tienen un incremento del riesgo de eventos aórticos en el embarazo, especialmente durante el tercer trimestre y el periodo posparto. Se debería valorar, en centros de referencia, la cirugía aórtica profiláctica pregestacional con diámetros aórticos ≥ 40mm. Es importante un diagnóstico precoz, un estudio pregestacional de toda la aorta, la administración de bloqueadores beta y un estrecho seguimiento durante el embarazo, especialmente durante el último trimestre y el posparto.

Palabras clave:
Síndrome de Marfan
Embarazo y puerperio
Disección aórtica
Diámetro aórtico

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